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Published on: May 17, 2017
Pediatric craniopharyngioma: when hypoglycemia reveals a brain tumor. Illustrative case
Oualid M Hmamouche1, Marouane Hammoud1, Faycal Lakhdar1
1Department of Neurosurgery, Hassan II University Hospital, Fez, Morocco.
Insights
Craniopharyngioma can rarely cause severe hypoglycemia and coma in children. Prompt endocrine evaluation and imaging are vital for early diagnosis and effective management of this rare presentation.
Area of Science:
- Pediatric Endocrinology
- Pediatric Neurosurgery
- Oncology
Background:
- Craniopharyngiomas (CPs) typically present with symptoms like intracranial hypertension, visual disturbances, or growth failure.
- Hypoglycemia as an initial symptom of craniopharyngioma is exceptionally rare, indicating hypothalamic-pituitary dysfunction.
Purpose of the Study:
- To highlight the rare presentation of craniopharyngioma with severe hypoglycemia.
- To emphasize the importance of prompt endocrine evaluation and imaging for unexplained pediatric hypoglycemia.
Main Methods:
- A case report of a 4-year-old girl presenting with coma and severe hypoglycemia.
- Diagnostic workup included endocrine testing and brain MRI.
- Sellar-suprasellar mass (craniopharyngioma) was surgically resected.
Main Results:
- The patient experienced recurrent hypoglycemia, central adrenal insufficiency, and hypothyroidism.
- Brain MRI revealed a cystic sellar-suprasellar mass.
- Histopathology confirmed adamantinomatous craniopharyngioma; postoperative hypoglycemia resolved with hormone replacement.
Conclusions:
- Craniopharyngioma can exceptionally present with severe recurrent hypoglycemia and coma in children.
- Prompt endocrine evaluation and sellar imaging are crucial for unexplained pediatric hypoglycemia.
- Multidisciplinary management involving neurosurgery and endocrinology is key for favorable outcomes.
Background:
Craniopharyngiomas (CPs) usually manifest with symptoms of intracranial hypertension secondary to obstructive hydrocephalus, visual disturbances, or growth failure. Hypoglycemia as the sentinel presentation is exceptionally rare and reflects hypothalamic-pituitary dysfunction.
Observations:
A previously healthy 4-year-old girl presented with progressive headache and vomiting for 4 days, followed by coma (Glasgow Coma Scale score 6). Emergency testing revealed profound hypoglycemia (capillary glucose 40 mg/dL [2.2 mmol/L]); consciousness returned rapidly after an intravenous dextrose bolus was administered. Because she had been vomiting, the hypoglycemia was initially attributed to malnutrition secondary to poor oral intake. However, recurrence of hypoglycemia within 24 hours prompted further evaluation. Abdominal CT excluded insulinoma and adrenal tumors. Endocrine testing revealed low morning cortisol and free thyroxine with inappropriately low thyroid-stimulating hormone, consistent with central adrenal insufficiency and hypothyroidism, while other hypothalamic-pituitary axes were preserved. Brain MRI showed a cystic sellar-suprasellar mass displacing the optic chiasm. Gross-total resection was achieved via a pterional approach. Histology confirmed an adamantinomatous CP. Postoperatively, hypoglycemia resolved, and the patient remained stable on hydrocortisone and levothyroxine replacement.
Lessons:
CP, although relatively frequent, may exceptionally present with severe recurrent hypoglycemia and coma. Unexplained pediatric hypoglycemia warrants prompt endocrine evaluation and sellar imaging. Early recognition and multidisciplinary management (neurosurgery and endocrinology teams) are crucial for favorable outcomes. https://thejns.org/doi/10.3171/CASE25727.
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